• Journal of Chest Surgery
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• v.14 no.3
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• pp.241-246
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• 1981

A series of 132 consecutive cases of secundum type atrial septal defect operated from 1963 to 1980 in the department of Thoracic Surgery of Seoul National University Hospital is analysed. The ages of the patients ranged from 12 months to 57 years and the ratio of men to women was 1 to 1.4. Clinical analysis revealed 33 patients [26%] were asymptomatic and 15 patients [12%]had congestive heart failure. Hemodynamic analysis revealed that severe pulmonary hypertension which is the most serious risk factor is ASD developed in 10 patients [9%] and pulmonary artery pressure significantly increased after the age of 20 years. There were 10 patients with sinus venosus defect and 7 patients with partial anomalous pulmonary venous return. The operative mortality was 2.2%.

• Journal of Chest Surgery
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• v.34 no.9
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• pp.672-679
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• 2001

Background: Surgical correction of partial anomalous pulmonary venous connection to the superior vena cava has been associated with postoperative venous obstruction and sinus node dysfunction. In this paper we describe our current approach and its short-term results. Material and Method: Between April 1999 and January 2000, 5 consecutive patients, ranging from 2 months to 66 years old, underwent corrective operation for partial anomalous pulmonary venous connection to the superior vena cava at Sejong General Hospital and Daegu Catholic University Medical Center. Surgical correction involved diversion of the pulmonary venous drainage to the left atrium using a right atrial flap(2 patients) or prosthetic patch(3 patients) with division of the superior vena cava superior to the restore site of the pulmonary veins and reimplantation on the right atrial appendage to restore systemic venous drainage. Result: All patients were discharged between postoperative day 9 and 15 without complications. One Russian boy returned to his country, therefore, he was lost to follow-up after discharge. Remaining 4 patients were asymptomatic and in normal regular sinus rhythm at a mean follow-up of 17.75$\pm$4.27 months. Follow-up echocardiographic study (range, 12 to 24 months) revealed no incidence of narrowing of the venous pathways or of residual shunt. Conclusion: Our current approach is relatively simple and reproducible in achieving unobstructive pulmonay venous and SVC pathways. By avoiding incision across the cavoatrial junction, surgical injury to the sinus node and its artery may be minimized. The presented surgical technique can be safely and effectively applied to the selected patients.

• Journal of the Korean Society of Radiology
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• v.81 no.6
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• pp.1523-1528
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• 2020

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

• Journal of Chest Surgery
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• v.25 no.4
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• pp.406-411
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• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.569-573
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• 2007

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The post-operative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.52-55
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• 2002

We report one case of an 18-day-old female patient, weighing 3.4 kg, with severe cyanosis. The diagnosis was made with only transthoracic echocardiography, which revealed cor triatriatum with an atretic small opening of fibromuscular membrane, obstructive infracardiac total anomalous pulmonary venous drainage(TAPVD), severely restrictive interatrial communication, and scanty mitral inflow and aortic forward flow. The preoperative decision-making for biventricular repair was not easy due to collapsed left heart system caused by remarkably reduced blood flow An emergent operation was performed due to severe cyanosis. All left heart structures were somewhat hypoplastic but thought to be adequate for systemic circulation. Biventricular repair was done without specific intraoperative problems. The postoperative course was uneventful. The patient has been doing well with no evidence of pulmonary vein stenosis or mitral regurgitation for 4 months after operation.

• Journal of Chest Surgery
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• v.24 no.1
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• pp.1-7
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• 1991

This report describes our 10-year experience with intracardiac repair in 42 patients older than 16 year with tetralogy of Fallot. The mean age was 22.0$\pm$5.18 years[range 16~41]. The preoperative clinical manifestations were cyanosis & clubbing[93%], frequent URI [55%], anoxic spell[40%], pulmonary tuberculosis[21%], tuberculous empyema[7%], chronic renal failure[7%], congestive heart failure[7%] and subacute bacterial endocarditis [2%], etc. The previous shunt procedure for palliation had been performed in 7 patients. The type of VSD were typical perimembranous type[67%], total canal defect[28%] and combined type[5%]. The right ventricular outflow tract obstruction were combined type[69%], infundibular type[21%] and valvular type[10%]. Transannular patch was used in 50% of patients. Associated cardiovascular anomalies were ASD[33%], PFO[31%], Rt. aortic arch[10%], Lt. SVC[10%], single Rt. pulmonary artery, single Lt. coronary artery, ASI, proximal stenosis of Rt. pulmonary artery and anomalous systemic venous return, etc. Hospital mortality was 7.1%[3 cases]in overall. The causes of hospital deaths were revealed low cardiac output & acute renal failure[2 cases], postoperative bleeding[1 case]. There were 2 late deaths 3 & 68 months after surgery. Residual intracardiac shunt was detected in 2 patients. one patient was successfully reoperated and another patient had Qp /Qs ratio less than 1.5.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.862-870
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• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.